Pediatric pulmonary sequestration

Pulmonary sequestration, also known as bronchopulmonary sequestration or BPS, is a condition in which abnormal tissue develops on a fetus’s lungs. The expert high-risk obstetricians, pediatric surgeons and neonatologists of the Center for Maternal/Fetal Health at Rocky Mountain Hospital for Children in Denver are specially trained to diagnose and treat this rare condition. In addition, our staff and facility are uniquely equipped to handle the careful treatment of your baby’s health during pregnancy, as well as during and after birth.

About pulmonary sequestration

Pulmonary sequestration is a congenital condition in which lung tissue forms abnormally. The tissue does not work like normal lung tissue and can steal blood flow that is necessary for lung function. Pulmonary sequestrations can be located next to the lung (extrapulmonary or extralobar sequestration) or in one part of the lung (intrapulmonary or intralobar sequestration). The mass develops without a connection to the baby’s airways and has its own blood vessels, which also have formed abnormally. This condition is similar to another fetal lung condition, CCAM/CPAM, but distinguishes itself with an artery that directly connects it to the aorta.

Pulmonary sequestration diagnosis

Fetal pulmonary sequestration typically is diagnosed during the 20-week ultrasound. To distinguish it from CCAM/CPAM, a color flow Doppler ultrasound can highlight blood supply. While the concern with CCAM/CPAM is size, the primary concern with pulmonary sequestration is blood flow.

Pulmonary sequestration treatment

Babies diagnosed with BPS will undergo regular ultrasounds during the remainder of the pregnancy to check the size, location and impact of the BPS. An estimated two-thirds of BPS lesions will shrink before birth and some may even become small enough that treatment after birth is not warranted. However, in rare cases, the mass can grow rapidly and push the heart and lungs to the other side of the chest leading to heart failure, determined by the accumulation of fluid in multiple areas of the body, called fetal hydrops.

If fetal hydrops occur, treatment is necessary during pregnancy. The pediatric surgeons of Rocky Mountain Pediatric Surgery are uniquely qualified to perform such delicate procedures.

Babies who do not develop fetal hydrops can be carried to term and delivered without complication. However, delivery at a hospital equipped with a Level IV Neonatal Intensive Care Unit (NICU) is crucial, as we are uniquely qualified to care for high-risk newborns and any potential complications that may arise. Typically, our specialized pediatric surgeons of Rocky Mountain Pediatric Surgery will schedule surgery to remove the BPS lesion when the baby is a few days old.

Our surgeons are highly experienced in minimally invasive (also called thoracoscopic surgery) techniques, even pioneering such techniques and treatments for babies and children. These techniques lessen the impact of surgery on the baby with smaller incisions, decreased pain, shorter recovery times and significantly less long-term musculoskeletal problems, such as scoliosis and chest wall deformity. To discuss diagnosis and treatment options, please call our Center for Maternal/Fetal Health team at (720) 754-7642.

Long-term outlook for pulmonary sequestration

Babies diagnosed with pulmonary sequestration can go on to live healthy, normal lives. Lungs continue to develop until about nine years old, so children have plenty of time to create normal, healthy lung tissue after the abnormality is removed.

To find an expert high-risk obstetrician or pediatric surgeon, contact our program care coordinator at (720) 754-7642.